title: Polyarteritis nodosa clinically mimicking nonocclusive mesenteric ischemia.
abstract: Here, we present the case of a 74-year-old Japanese man with segmental intestinal necrosis, which developed after treatment with pulsed methylprednisolone for mononeuritis multiplex. The patient was weakly positive for myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA). Computed tomography and surgical findings were compatible with nonocclusive mesenteric ischemia (NOMI). He underwent small intestinal resection by emergency surgery and an intestinal fistula was made. Pathologically, necrotizing vasculitis with fibrinoid necrosis was present in medium to small-sized arteries, which was equivalent to Arkin's classification II-IV. Most of the arteries had fibrous intimal thickening, which was considered to obstruct the arteries and thus cause segmental intestinal necrosis. A diagnosis of polyarteritis nodosa (PAN) was made, and intravenous cyclophosphamide pulse therapy was added to the therapeutic regimen. This patient was successfully treated with these multidisciplinary therapies and his stoma was finally closed. This is a very rare and indicative case of PAN weakly positive for MPO-ANCA and clinically mimicking NOMI, which occurred even after treatment with pulsed methylprednisolone.
authors.affiliation: Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan. tsuyoshirajp@med.tohoku.ac.jp
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Shirai
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Fujii
authors.forename: Shinichiro
authors.initials: S
authors.lastname: Saito
authors.forename: Tomonori
authors.initials: T
authors.lastname: Ishii
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Yamaya
authors.forename: Shigehito
authors.initials: S
authors.lastname: Miyagi
authors.forename: Satoshi
authors.initials: S
authors.lastname: Sekiguchi
authors.forename: Naoki
authors.initials: N
authors.lastname: Kawagishi
authors.forename: Masato
authors.initials: M
authors.lastname: Nose
authors.forename: Hideo
authors.initials: H
authors.lastname: Harigae
mesh_terms: D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D015415:Biomarkers / Q000097:blood; D003520:Cyclophosphamide / Q000008:administration & dosage; D003937:Diagnosis, Differential; D005355:Fibrosis; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007422:Intestines / Q000098:blood supply / Q000473:pathology / Q000601:surgery; D007511:Ischemia / Q000175:diagnosis*; D008297:Male; D065666:Mesenteric Ischemia; D009336:Necrosis; D010488:Polyarteritis Nodosa / Q000097:blood / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D011237:Predictive Value of Tests; D020551:Pulse Therapy, Drug; D054047:Surgical Stomas; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014652:Vascular Diseases / Q000175:diagnosis*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D015415:Biomarkers; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide
keywords: Anti-neutrophil cytoplasmic antibody; Intestinal necrosis; Myeloperoxidase; Nonocclusive mesenteric ischemia; Polyarteritis nodosa